进行性系统性硬皮病与多发性肌炎重叠综合征40例报告

Progressive Systemic Sclerosis and Polymyositis Overlap Syndrome: A Report of 40 Cases

系统性硬皮病(progressive systemic sclerosis,PSS)与多发性肌炎(polymyositis PM)重叠综合征(PSS-PMOS)40例有典型PSS和PM表现,均有雷诺氏现象,多有弥漫性全身皮肤硬化;均有肌无力,肌酶谱升高,肌电图示肌源性损害,组织病理示肌炎改变。有多系统损害表现,消化系统相循环系统损害较严重。血清学检查常有多种自身抗体。糖皮质激素并中药治疗反应较好。只要对本病有足够警惕,仔细观察病情表现及发展变化,能够做到早期正确诊断。

We studied 40 patients who had clinical features of both typical progressive systemic sclerosis (PSS) and polymyositis (PM). The indurated skin extendede over the metacarpophalangeal joints in all patients. The sclerotic changes, diffuse in type, involved the whole body, including the face, neck, extremities and trunk in most cases. All showed Raynaud's phenomenon. All patients had muscle weakness, elevated skeletal muscle enzymes and myogenic disorders in electromyograms, and biopsy of muscles showed myositic changes. The symptoms involved multiple systems, particularly the gastrointestinal and circulatory systems, presenting features of both PSS and PM. Serologic examinations often revealed auto-antibodies in these patients. Treatment with small doses of corticosteroid and traditional chinese medicines usually had good results. We found that either PSS or PM might appear first, or they might appear simultaneously. It is our opinion that as long as we keep alert and carefully observe the features and their development, we can easily diagnose the disease early.